In some children with
bilateral Wilms' tumor, reduction of
tumor burden cannot be accomplished without total
nephrectomy. In
Denys-Drash syndrome,
nephrectomy is required for associated
Wilms' tumor or after progression to
end stage renal disease secondary to
diffuse mesangial sclerosis because of risk of development of
Wilms' tumor. Current recommendation is to wait at least 1-2 yr after completion of
chemotherapy for
Wilms' tumor before
renal transplantation. The North American Pediatric Renal Transplant Cooperative Study dialysis (1992-2001) and transplant registries (1987-2002) were analyzed, comparing children 0-18 yr old with
Wilms' tumor and
Denys-Drash syndrome to other primary diagnoses. There were 37 children with
Wilms' tumor and 33 with
Denys-Drash syndrome in the dialysis registry. Of these, 10 children with
Wilms' tumor and three with
Denys-Drash syndrome did not receive a renal transplant and all died. The cause of death was
Wilms' tumor in eight children with
Wilms' tumor and in one with
Denys-Drash syndrome. The transplant registry included 43 children with
Wilms' tumor, 43 children with
Denys-Drash syndrome, and 7469 patients with other diagnoses. Acute rejection, graft and patient survival profiles from all three groups at 6 months, 1 and 3 yr post-transplant were comparable. There were no graft failures or deaths because of recurrent
Wilms' tumor in the Drash group. There was one death with
Wilms' tumor in the Wilms' group - a 2.5-yr-old child transplanted after 6 months of dialysis who died of Wilms' <6 months after
renal transplantation. In conclusion, most children dialyzed because of
Wilms' tumor and
Denys-Drash syndrome who did not receive a renal transplant died of
Wilms' tumor. However, the outcomes of children with
Wilms' tumor and
Denys-Drash syndrome who proceeded to
renal transplantation are comparable with children with other diagnoses, with no graft failures because of recurrence and only one death from
Wilms' tumor in a Wilms' patient who received only a short course of dialysis prior to
transplantation. Current practices in children with
Wilms' tumor and
Denys-Drash syndrome appear to be on target to portend good outcome following
renal transplantation.